M S Ren1, Y X Fan1, R M Yang1, Y Z Han1, G J Wu1, Y R Xin1, L Yu1. 1. Ming-Shan Ren, Ren-Min Yang, Yong-Zhu Han, Institute of Neurology, Teaching Hospital, Anhui College of TCM, Hefei 230031, Anhui Province, China.
Abstract
AIM: To further explore the etiological mechanism of Wilson's disease (WD) by comparing the changes of biliary trace elements and its clinical phenotype. METHODS: WD patients with different types and conditions (n = 20), non-WD patients with chronic liver damage (n = 22), and healthy volunteers (n = 10; used as controls) were studied. Biliary samples were taken by duodenal drainage. Atom absorption spectrophotometer was used to assay the copper and zinc content of each sample. RESULTS: In WD, the copper content and copper/zinc ratio of biliary juice were evidently lower than those of non-WD patients with chronic liver damage and of healthy controls (F = 14.76, 25.4; 14.92, 26.2 respectively; P < 0.01), while the biliary zinc level had no significant difference from the two non-WD control groups (P > 0.05). There were significant differences in biliary copper excretion among patients with different types and conditions (F = 3.75, P < 0.05; F = 6.20, P < 0.01). CONCLUSION: Copper excretion by liver and the biliary system decreases obviously in WD, which plays a key role in the phenotypic copper retention, and the biliary copper retention is closely related with the severity of hepatic injury and illness.
AIM: To further explore the etiological mechanism of Wilson's disease (WD) by comparing the changes of biliary trace elements and its clinical phenotype. METHODS:WDpatients with different types and conditions (n = 20), non-WDpatients with chronic liver damage (n = 22), and healthy volunteers (n = 10; used as controls) were studied. Biliary samples were taken by duodenal drainage. Atom absorption spectrophotometer was used to assay the copper and zinc content of each sample. RESULTS: In WD, the copper content and copper/zinc ratio of biliary juice were evidently lower than those of non-WDpatients with chronic liver damage and of healthy controls (F = 14.76, 25.4; 14.92, 26.2 respectively; P < 0.01), while the biliary zinc level had no significant difference from the two non-WD control groups (P > 0.05). There were significant differences in biliary copper excretion among patients with different types and conditions (F = 3.75, P < 0.05; F = 6.20, P < 0.01). CONCLUSION:Copper excretion by liver and the biliary system decreases obviously in WD, which plays a key role in the phenotypic copper retention, and the biliary copper retention is closely related with the severity of hepatic injury and illness.
Authors: R E Tanzi; K Petrukhin; I Chernov; J L Pellequer; W Wasco; B Ross; D M Romano; E Parano; L Pavone; L M Brzustowicz Journal: Nat Genet Date: 1993-12 Impact factor: 38.330
Authors: Kamil Janowski; Elizabeth Shumbayawonda; Matt Kelly; Carlos Ferreira; Maciej Pronicki; Wieslawa Grajkowska; Magdalena Naorniakowska; Piotr Pawliszak; Sylwia Chełstowska; Elżbieta Jurkiewicz; Rajarshi Banerjee; Piotr Socha Journal: Children (Basel) Date: 2022-04-25